Susac综合征--附二例报告

目的：讨论Susac综合征的临床表现及诊断。方法：对2例Susac综合征进行临床及辅助检查资料分析。结果：Susac综合征好发于青年女性,表现为急性、亚急性起病的多发、弥散神经症状、听力下降和视野缺损。典型表现：大脑MRI T2加权相显示白质及灰质内大量小灶增强信号、视网膜动脉闭塞、中到低频单侧或双侧感音性耳聋。结论：Susac综合征容易误诊,通过仔细询问病史及体格检查并结合辅助检查可以在疾病的早期阶段确诊。MRI、单光子发射计算机体层摄影术、视网膜荧光血管造影及听力测定有助于诊断。

Susac syndrome: a report of two cases

Objective To investigate the clinic characters and diagnosis of Susac syndrome. Methods Two cases were systematically studied by the authors. Results All the two cases were young women. The clinical manifestations include acute and subacute multifocal and diffuse encephalopathic symptoms, hearing loss, and visual loss. Diagnosis is facilitated by demonstration of retinal arteriolar occlusions without uveitis or keratoconjunctivitis, mid-to-low frequency unilateral or bilateral sensorineural hearing loss, and numerous small foci of increased signal in the white and gray matter on T2-weighted brain magnetic resonance imaging. Conclusions This rare syndrome often can be identified at an early stage with a careful history and physical examination. MRI, SPECT, retinal fluorescein angiography and audiometry will contribute to diagnosis.